WORLD JOURNAL OF ADVANCE
HEALTHCARE RESEARCH

Dr. Aamir Malik* and Neha Sharma

ABSTRACT

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a hyper-inflammatory syndrome characterized by fever, hepatosplenomegaly, bone marrow, skin, and central nervous system infiltration. Incidence of HLH is 1 per 50,000 live births worldwide. In this case series, we present 13 cases of HLH in adults; their presentation, course, and outcome over a period of three years Materials and Methods: We retrospectively looked at 13 cases of HLH who presented to our hospital. Data was collected from computerised electronic medical records in addition to referring to hard copies of patients’ records. The median age at diagnosis was 42.5 years (age range 25 years–60 years). Diagnosis was based on fulfilling clinical and laboratory criteria. Results: All patients initially presented with unremitting prolonged fever, splenomegaly and bicytopenia or pancytopenia. Blood investigations showed elevated ferritin (n = 13), elevated liver enzymes[6] and high lactate dehydrogenase (LDH),[8] hypertriglyceridemia and hypofibrinogenemia in (n = 10). The main causes were Epstein barr virus,[5] malignancy(b cell lymphoma),[3] abdomen tuberculosis[2] Conclusions: HLH is a rare but rapidly fatal disease and may present in a wide range of different presentations. A persistent and prolonged fever with associated progressive abdominal distention (hepatosplenomegaly) and cytopenias over an acute phase should raise the suspicion of HLH. Accordingly, early diagnosis and prompt aggressive treatment are vital for patients’ survival and favorable outcome.

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